Association between bosentan and reduced detection of precapillary pulmonary hypertension in systemic sclerosis: evidence from a EUSTAR study
Year: 2026
Type: article
Abstract: Objectives: To evaluate whether exposure to vasoactive-vasodilating drugs (VVDs), particularly bosentan, is associated with reduced detection of precapillary pulmonary hypertension (PH) in systemic sclerosis (SSc). Design: Post hoc retrospective case-control study. Setting: European Scleroderma Trials and Research database. Participants: SSc patients who underwent their first right heart catheterisation (RHC); patients with postcapillary PH (pulmonary wedge pressure >15 mm Hg) were excluded. Interventions: Exposure to VVDs (endothelin receptor antagonists (ERAs), phosphodiesterase-5 inhibitors, prostanoids) was defined as 'ever' before or 'ongoing' at the time of RHC. Main outcome measures: Presence of precapillary PH at RHC. Results: Among the 944 patients included in the study, 498 (53%) were diagnosed with precapillary PH. Bosentan 'ever' was associated with a lower likelihood of a precapillary PH detection (OR 0.594, 95% CI 0.362 to 0.975) in the whole population. When considering 'ongoing' bosentan exposure, this result was confirmed only in patients with current DU (OR 0.230, 95% CI 0.081 to 0.652). ERAs in general showed a trend for protective effects towards detection of precapillary PH in patients with current DU ('ever': OR 0.636, 95% CI 0.349 to 1.158; 'ongoing': OR 0.435, 95% CI 0.193 to 1.064), while other VVD classes did not. Conclusions: Bosentan, especially when employed in patients with current DU at the time of RHC, was associated with less detection of precapillary PH. These findings highlight the need for individualised treatment strategies and further research to optimise the timing and patient selection for VVD therapy in SSc.
Authors: Nicola Farina, Silvia Bellando-Randone, Sebastien Sanges, Hilde Jenssen Bjørkekjaer, Lorenzo Tofani, Marie-Elise Truchetet, Vanessa Smith, Andra Balanescu, Christina Bergmann, Yannick Allanore, Philipp Klemm, Simona Truglia, Luca Idolazzi, Christopher Denton, Roberta Foti, Anna Wojteczek, Emmanuel Chatelus, Madelon Vonk, Serena Guiducci, David Launay, Eric Hachulla, Jeska K de Vries-Bouwstra, Anna-Maria Hoffmann-Vold, Oliver Distler, Marco Matucci-Cerinic, Cosimo Bruni
Institutions: Azienda Ospedaliero-Universitaria Careggi, IRCCS Ospedale San Raffaele, IRCCS San Raffaele Roma, University of Florence, Inserm, Université de Lille, Centre Hospitalier Universitaire de Lille, Institute for Translational Research in Inflammation, Hospital of Southern Norway, Centre Hospitalier Universitaire de Bordeaux, Ghent University, Carol Davila University of Medicine and Pharmacy, Universitätsklinikum Erlangen, Université Paris Cité, Hôpital Cochin, Justus-Liebig-Universität Gießen, Sapienza University of Rome, University of Verona, The Royal Free Hospital, University College London, Azienda Ospedaliero Universitaria Policlinico "G.Rodolico - San Marco", Gdańsk Medical University, Hôpitaux Universitaires de Strasbourg, Radboud University Nijmegen, Radboud University Medical Center, Leiden University Medical Center, Oslo University Hospital, University of Zurich, University Hospital Zurich
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $4,150
Funders: Univerzita Karlova v Praze, University of Bern, Università degli Studi di Cagliari, University of Occupational and Environmental Health, Inselspital, Universitätsspital Bern, National Institute for Health and Care Research, Ege Üniversitesi, KU Leuven, Universität zu Lübeck, Sapienza Università di Roma, National and Kapodistrian University of Athens, Hokkaido University, Hôpitaux Universitaires de Genève, Centre Hospitalier Universitaire Vaudois, Università degli Studi di Brescia, Medicinski Fakultet, Sveučilište u Zagrebu, Universitatea de Medicină şi Farmacie "Carol Davila" Bucureşti, Debreceni Egyetem, Pécsi Tudományegyetem, Uniwersytet Medyczny w Lublinie, Skånes universitetssjukhus, Bispebjerg Hospital, Centre Hospitalier Universitaire de Poitiers, School of Medicine, Stanford University
OpenAlex ID: W7225078153